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Abstract

Congenital synagthia is a rare craniofacial anomaly characterized by the abnormal fusion of the maxilla and mandible, which can lead to severe complications if not diagnosed early. A case of a 13-hour-old male neonate with respiratory distress and associated anomalies was presented, highlighting the need for surgical intervention and the potential link to Pierre Robin Sequence. The case emphasizes the importance of early diagnosis and intervention, as well as the necessity for thorough evaluation of associated conditions.

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drannybassey
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3 views

Abstract

Congenital synagthia is a rare craniofacial anomaly characterized by the abnormal fusion of the maxilla and mandible, which can lead to severe complications if not diagnosed early. A case of a 13-hour-old male neonate with respiratory distress and associated anomalies was presented, highlighting the need for surgical intervention and the potential link to Pierre Robin Sequence. The case emphasizes the importance of early diagnosis and intervention, as well as the necessity for thorough evaluation of associated conditions.

Uploaded by

drannybassey
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as DOCX, PDF, TXT or read online on Scribd
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Here's the complete abstract:

Abstract

Background:
Congenital synagthia, the abnormal fusion of the maxilla and mandible, is a rare craniofacial
anomaly that can be fibrous (synechiae) or bony (synostosis). Its rarity, variable presentation,
and association with syndromic anomalies make standardized classification and management
challenging. Early diagnosis is critical, as the condition may result in life-threatening feeding and
respiratory complications. This case highlights the clinical relevance of early surgical
intervention and the potential association with Pierre Robin Sequence.

Case Presentation:
A 13-hour-old male neonate presented with respiratory distress, poor cry at birth, and tongue
deformity. He was delivered at term via emergency cesarean section for fetal distress.
Examination revealed a thick fibrous band connecting the floor of the mouth to the hard palate
(synechiae), ankyloglossia, a high-arched palate, micrognathia, glossoptosis, and an Epstein
pearl. A provisional diagnosis of congenital synagthia was made. Echocardiography also
showed a patent ductus arteriosus. On day 2 of life, he underwent surgical excision of the
fibrous band and lingual frenectomy. Intraoperatively, findings confirmed fibrous fusion of the
jaws with associated craniofacial anomalies. Postoperatively, the baby experienced feeding
difficulties and intermittent respiratory distress, prompting a clinical diagnosis of Pierre Robin
Sequence. Genetic testing was not performed due to resource limitations. The baby recovered
and was discharged on day 12 of life but was unfortunately lost to follow-up.

Conclusion:
This case underscores the importance of early identification and prompt intervention in
congenital synagthia to prevent life-threatening complications. The clinical overlap with Pierre
Robin Sequence emphasizes the need for thorough evaluation of associated anomalies. Due to
its rarity and the lack of a universally accepted classification system, reporting such cases adds
to the growing body of knowledge and may aid in shaping future diagnostic and management
protocols. Long-term follow-up is essential for monitoring development and improving
understanding of this complex anomaly.

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